CARDIAC OPERATIONS IN CHILDREN WITH MARFANS-SYNDROME - INDICATIONS AND RESULTS

Background. The development of new screening techniques for the early detection of Marfan's syndrome has prompted evaluation of the results of cardiac operations in children with this syndrome. The purpose of t his study was to determine the surgical indications, operative results , and need for reoperation in children with Marfan's syndrome. Methods . From 1980 to 1996, 245 patients underwent cardiac operations for com plications of Marfan's syndrome; 26 (11%) were less than 18 years of a ge. The mean age at the time of operation was 10.3 +/- 1 years (range, 8 months to 17 years); 18 of the patients were male. Indications for operation were aortic root dilatation (15 patients), mitral regurgitat ion (4 patients), aortic root dilatation and mitral regurgitation (6 p atients), and aortic arch aneurysm (1 patient). Operations included ao rtic root replacement (15 patients), aortic root replacement and mitra l repair (5 patients), aortic root replacement and mitral replacement (1 patient), mitral repair (3 patients), mitral replacement (1 patient ), and arch aneurysm repair (1 patient). The mean aortic root diameter in patients undergoing aortic root replacement was 6.2 +/- 0.2 cm. On ly 1 patient underwent ascending aortic dissection. Results. There wer e no operative deaths. At a mean follow-up of 67.1 +/- 10.2 months, 8 patients required a second cardiac procedure (41% +/- 17% 10-year free dom from reoperation). Indications for further operations were distal aortic pathology (3 patients), aortic root dilatation after initial mi tral operation (3 patients), failed mitral repair (1 patient), and hom ograft degeneration (1 patient). Risk factors for a second cardiac pro cedure were age less than 10 years at the time of the first operation (p < 0.003) and mitral regurgitation (p < 0.04). Overall, 25 (96%) of 26 patients have undergone aortic root replacement and 11 (42%) patien ts have undergone a mitral procedure. There have been 4 late deaths, a ll of presumed cardiac origin. The 10-year survival rate is 79% +/- 10 %. All surviving patients are in New York Heart Association functional class I or II. Conclusions. We conclude that (1) aortic root dilatati on is the most common surgical indication in children with Marfan's sy ndrome, (2) mitral regurgitation is the second most common indication, (3) aortic dissection is unusual in children with Marfan's syndrome, and (4) careful follow-up is necessary, particularly in younger childr en, because more than half of all children with Marfan's syndrome requ ire repeated cardiac operations within 10 years. (C) 1997 by The Socie ty of Thoracic Surgeons.